Clinicoradiologic Correlation in 22 Egyptian Children With Megalencephalic Leukoencephalopathy With Subcortical Cysts.

Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare genetic form of cerebral white matter disease whose clinicoradiologic correlation has not been completely understood. In this study, we investigated the association between clinical and brain magnetic resonance imaging (MRI) features in 22 Egyptian children (median age 7 years) with MLC. Gross motor function was assessed using the Gross Motor Function Classification System, and evaluation of brain MRI followed a consistent scoring system. Each parameter of extensive cerebral white matter T2 hyperintensity, moderate-to-severe wide ventricle/enlarged subarachnoid space, and greater than 2 temporal subcortical cysts was significantly associated (P < .05) with worse Gross Motor Function Classification System score, language abnormality, and ataxia. Having >2 parietal subcortical cysts was significantly related to a worse Gross Motor Function Classification System score (P = .04). The current study indicates that patients with MLC manifest signification association between certain brain MRI abnormalities and neurologic features, but this should be confirmed in larger studies.

Orbitocranial Fibrous Dysplasia: Outcome of Radical Resection and Immediate Reconstruction With Titanium Mesh and Pericranial Flap.

INTRODUCTION: Fibrous dysplasia (FD) is a non-neoplastic developmental fibro-osseous disease. It represents 2.5% of all bone tumors and 5% to 7% of the benign bone tumors. Orbitocranial region is involved in about 20% of the patients. The main presentations are craniofacial deformity and headache. Loss of vision is the most devastating result of this disease. There is no medical treatment to cure or prevent FD. Radiation therapy is contraindicated. Surgery for the orbitocranial FD is often challenging because of the proximity of neurovascular and ocular structures. Conservative surgical shaving and recontouring is always associated with suboptimal results. Radical excision is potentially curative with no extra morbidity. Orbital hypertelorism, dystopia, or proptosis can be corrected only by radical excision and reconstruction. AIM: The aim of the study was to evaluate the outcome of radical excision of the orbitocranial FD and immediate reconstruction using titanium mesh and pericranial flap. PATIENTS AND METHODS: This prospective study had been conducted on 22 patients with orbitocranial FD with age range from 17 to 52 years (mean 29.5). Radical excision of the lesions was done for all patients through transcranial approach. Immediate reconstruction was achieved using titanium mesh and pericranial flap. RESULTS: Intraoperative dural tears and cerebrospinal fluid leak were reported in 2 patients and repaired with galeal graft. Supraorbital anesthesia occurred in 6 patients. Of these, 2 patients were transient, while the remaining 4 patients were permanent. Wound infection was noticed in 1 patient who improved by medical treatment. Temporary postoperative diplopia occurred in 1 patient and temporary postoperative impaired vision in 1 other patient. In all patients, acceptable or good aesthetic results were observed. No recurrence was detected in our series during the follow-up period that ranged from 24 to 58 months (mean 37.5 months). CONCLUSION: Radical excision of orbitocranial FD is potentially curative with no extra morbidity. It can achieve good aesthetic and functional results with no recurrence.